What constitutes a severe acute porphyria attack?

Severe attack features: severe abdominal pain requiring IV opioids, peripheral neuropathy (motor weakness), psychiatric manifestations, SIADH with Na 120 bpm, or respiratory compromise. Severe attacks require IV heme arginate (3 mg/kg/day × 4 days) started promptly.

What precipitants should be identified in acute porphyria attacks?

Major precipitants: drugs inducing CYP450 enzymes (listed at drugs-porphyria.org), caloric restriction, sex hormones (perimenstrual attacks), alcohol, stress, and infections. Urine PBG >6× ULN during attack confirms AHP diagnosis.

Porphyria Acute Attack Severity Assessment

Assess severity of acute hepatic porphyria attacks (AHP) including abdominal pain, neurological features, hyponatremia, and SIADH to guide heme arginate or givosiran therapy decisions.

Use the full interactive calculator below, or

Porphyria Acute Attack Severity Assessment

Frequently Asked Questions

What constitutes a severe acute porphyria attack?

What precipitants should be identified in acute porphyria attacks?